Applications

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application

Abreviews

Notes

WB

Use a concentration of 1 µg/ml. Predicted molecular weight: 54 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

ELISA

Use at an assay dependent concentration.

Titre using peptide based assay, 1:312500.

Target

FunctionAlpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins.

Involvement in diseaseDefects in FUCA1 are the cause of fucosidosis (FUCA1D) [MIM:230000]. FUCA1D is an autosomal recessive lysosomal storage disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas.